Cargando…

Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease

Autoantibodies to the insulin receptor are rare and typically cause severe insulin resistance and hyperglycemia, a condition termed type B insulin resistance. Uncommonly, antibodies to the insulin receptor can cause hypoglycemia. We present the case of a woman who developed recurrent severe hypoglyc...

Descripción completa

Detalles Bibliográficos
Autores principales: Petersen, Max C, Graves, Jonah M, Yao, Tony, Schomburg, Lutz, Minich, Waldemar B, Parks, Deborah L, McGill, Janet B, Salam, Maamoun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8668203/
https://www.ncbi.nlm.nih.gov/pubmed/34913021
http://dx.doi.org/10.1210/jendso/bvab182
_version_ 1784614519206576128
author Petersen, Max C
Graves, Jonah M
Yao, Tony
Schomburg, Lutz
Minich, Waldemar B
Parks, Deborah L
McGill, Janet B
Salam, Maamoun
author_facet Petersen, Max C
Graves, Jonah M
Yao, Tony
Schomburg, Lutz
Minich, Waldemar B
Parks, Deborah L
McGill, Janet B
Salam, Maamoun
author_sort Petersen, Max C
collection PubMed
description Autoantibodies to the insulin receptor are rare and typically cause severe insulin resistance and hyperglycemia, a condition termed type B insulin resistance. Uncommonly, antibodies to the insulin receptor can cause hypoglycemia. We present the case of a woman who developed recurrent severe hypoglycemia and myopathy, was found to have insulin receptor autoantibodies and mixed connective tissue disease, and had resolution of hypoglycemia with immunosuppression. A 55-year-old woman with a history of obesity, hypertension, and prior hemorrhagic stroke presented with recurrent severe hypoglycemia. A diagnostic fast resulted in hypoinsulinemic hypoketotic hypoglycemia. Adrenal function was intact. Progressive myopathy had developed simultaneously with her hypoglycemia, and rheumatologic evaluation revealed mixed connective tissue disease. The plasma acylcarnitine profile was normal, extensive oncologic evaluation including insulin-like growth factor 2 measurement was unrevealing, and anti-insulin antibody testing was negative. Ultimately, anti-insulin receptor antibodies were found to be present. The patient was treated with glucocorticoids and rituximab. Eight weeks after initiation of immunosuppression, the insulin receptor antibody titer had decreased and hypoglycemia had resolved. Eight months after diagnosis, the patient remained free of severe hypoglycemia despite tapering of glucocorticoids to a near-physiologic dose. Though antibodies to the insulin receptor typically cause severe insulin resistance, this patient had no evidence of insulin resistance and instead presented with recurrent severe hypoglycemia, which responded to glucocorticoids and rituximab. The diagnosis of insulin receptor antibody–mediated hypoglycemia is rare but should be considered in patients with systemic autoimmune disease, including mixed connective tissue disease, in the appropriate clinical context.
format Online
Article
Text
id pubmed-8668203
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher Oxford University Press
record_format MEDLINE/PubMed
spelling pubmed-86682032021-12-14 Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease Petersen, Max C Graves, Jonah M Yao, Tony Schomburg, Lutz Minich, Waldemar B Parks, Deborah L McGill, Janet B Salam, Maamoun J Endocr Soc Case Report Autoantibodies to the insulin receptor are rare and typically cause severe insulin resistance and hyperglycemia, a condition termed type B insulin resistance. Uncommonly, antibodies to the insulin receptor can cause hypoglycemia. We present the case of a woman who developed recurrent severe hypoglycemia and myopathy, was found to have insulin receptor autoantibodies and mixed connective tissue disease, and had resolution of hypoglycemia with immunosuppression. A 55-year-old woman with a history of obesity, hypertension, and prior hemorrhagic stroke presented with recurrent severe hypoglycemia. A diagnostic fast resulted in hypoinsulinemic hypoketotic hypoglycemia. Adrenal function was intact. Progressive myopathy had developed simultaneously with her hypoglycemia, and rheumatologic evaluation revealed mixed connective tissue disease. The plasma acylcarnitine profile was normal, extensive oncologic evaluation including insulin-like growth factor 2 measurement was unrevealing, and anti-insulin antibody testing was negative. Ultimately, anti-insulin receptor antibodies were found to be present. The patient was treated with glucocorticoids and rituximab. Eight weeks after initiation of immunosuppression, the insulin receptor antibody titer had decreased and hypoglycemia had resolved. Eight months after diagnosis, the patient remained free of severe hypoglycemia despite tapering of glucocorticoids to a near-physiologic dose. Though antibodies to the insulin receptor typically cause severe insulin resistance, this patient had no evidence of insulin resistance and instead presented with recurrent severe hypoglycemia, which responded to glucocorticoids and rituximab. The diagnosis of insulin receptor antibody–mediated hypoglycemia is rare but should be considered in patients with systemic autoimmune disease, including mixed connective tissue disease, in the appropriate clinical context. Oxford University Press 2021-11-28 /pmc/articles/PMC8668203/ /pubmed/34913021 http://dx.doi.org/10.1210/jendso/bvab182 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Petersen, Max C
Graves, Jonah M
Yao, Tony
Schomburg, Lutz
Minich, Waldemar B
Parks, Deborah L
McGill, Janet B
Salam, Maamoun
Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease
title Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease
title_full Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease
title_fullStr Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease
title_full_unstemmed Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease
title_short Insulin Receptor Autoantibody–mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease
title_sort insulin receptor autoantibody–mediated hypoglycemia in a woman with mixed connective tissue disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8668203/
https://www.ncbi.nlm.nih.gov/pubmed/34913021
http://dx.doi.org/10.1210/jendso/bvab182
work_keys_str_mv AT petersenmaxc insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease
AT gravesjonahm insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease
AT yaotony insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease
AT schomburglutz insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease
AT minichwaldemarb insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease
AT parksdeborahl insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease
AT mcgilljanetb insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease
AT salammaamoun insulinreceptorautoantibodymediatedhypoglycemiainawomanwithmixedconnectivetissuedisease